Since the first report of living donor liver transplantation (LDLT) in 1990,1 LDLT has quickly developed into a mainstream procedure for patients with end-stage liver disease because of organ shortages. However, with the increase in the number of LDLT procedures performed worldwide each year, problems associated with small-for-size living donor grafts have gradually surfaced. When a patient with an undersized partial graft suffers from persistent portal hyperperfusion, small-for-size syndrome (SFSS) may develop and cause disruption of liver regeneration and hepatic dysfunction.2, 3 Early treatment for SFSS is often required in order to prevent graft failure and retransplantation. Until now, the treatment options for SFSS have been very limited,4 and the most established method for the treatment of SFSS is the reduction of the portal flow via the embolization of a portion of the splenic blood flow (partial splenic embolization).5 However, if SFSS occurs in a patient who underwent splenectomy before transplantation, partial splenic embolization will not be feasible, and an alternative treatment option may be required. Transjugular intrahepatic portosystemic shunt (TIPS) placement is a well-established and minimally invasive procedure that may improve portal hypertension.6 Nonetheless, its application to the treatment of SFSS has rarely been reported.7 Here we describe a patient with SFSS after LDLT who had undergone splenectomy before transplantation and was in the end successfully treated with a TIPS implant.
Small-for-size syndrome (SFSS) is a serious complication after living donor liver transplantation (LDLT) that can disrupt liver regeneration and result in hepatic dysfunction. Until now, the treatment options for SFSS after LDLT have been very limited. Here we describe a patient with SFSS after LDLT who was successfully treated with a transjugular intrahepatic portosystemic shunt (TIPS). A 56-year-old man who had undergone adult-to-adult LDLT because of decompensated liver cirrhosis started displaying signs of acute jaundice and ascites within 72 hours of the operation. The patient was diagnosed with SFSS, and because he had already undergone splenectomy before the transplant, partial splenic embolization was not feasible. Consequently, the TIPS procedure was chosen in an attempt to reduce portal hyperperfusion. After the procedure, the patient's symptoms were gradually ameliorated and were eventually resolved. In conclusion, when partial splenic embolization is not feasible, TIPS placement may be a feasible option for the treatment of SFSS after LDLT. Liver Transpl, 2012. © 2012 AASLD.
A 56-year-old man with chronic hepatitis B–induced cirrhosis complicated by refractory ascites had suffered from repeated episodes of spontaneous bacterial peritonitis for 4 months. He was referred to be considered for liver transplantation, and his 48-year-old brother, who had a clean medical history, decided to donate a portion of his liver. Ten years before transplantation, the patient had undergone splenectomy and portal azygous disconnection because of hypersplenism and bleeding esophageal varices. LDLT was performed with a right lobe graft without the middle hepatic vein. The recipient's body weight was 72.4 kg, and the graft weighed 615 g, which was 47.5% of the recipient's standard liver weight and 0.85% of his body weight. The right hepatic vein graft was anastomosed to the inferior vena cava in an end-to-side fashion. In addition, the major tributaries of the middle hepatic vein from segments V and VIII were implanted directly into the recipient's inferior vena cava with a frozen grafted vein used as an interpositional vascular graft. The cold ischemia time of the graft was approximately 35 minutes. At the end of the operation, the graft had uniform perfusion, and a routine Doppler ultrasound study showed that all vessels, including the implanted V and VIII veins, were patent. However, within 72 hours of the operation, the patient started displaying signs of progressive graft dysfunction, which was characterized by acute jaundice and ascites. Repeated Doppler ultrasound examinations of the graft and the surrounding vessels did not find anything unusual except for the abnormal portal vein flow rate, which was 10 cm/second. Subsequently, liver biopsy was performed. Frozen sections did not show signs of graft rejection, but a paraffin section showed centrilobular sinusoidal dilatation and lobular hepatocanalicular cholestasis (Fig. 1). These findings were largely consistent with SFSS, but because the patient had already undergone splenectomy before transplantation, a conservative treatment was adopted. However, the patient's serum bilirubin level and ascites continued to increase, and within 1 month of the operation, mild hepatic encephalopathy and coagulopathy (international normalized ratio = 1.8) had also developed. Consequently, TIPS placement was discussed for the treatment of SFSS, and both the patient and the referring physician agreed to proceed. Thirty-five days after transplantation, TIPS placement was performed under intravenous conscious sedation. The procedure was performed from the right internal jugular vein with a transjugular liver access set (RUPS-100, Cook, Bloomington, IN). The preliminary caval pressure was measured to be 1.5 mm Hg. The right hepatic vein was catheterized, and the intrahepatic right anterior branch of the right portal vein was successfully punctured. The initial portal vein pressure was measured to be 28.7 mm Hg. The intrahepatic tract was dilated with a 6 mm × 60 mm balloon (Cordis, Roden, the Netherlands), and an 8 mm × 50 mm wall graft covered stent (Boston Scientific, Galway, Ireland) was implanted. A final portography examination was performed, and it confirmed that the shunt was functional (Fig. 2). The portal pressure was reduced to 15.4 mm Hg after TIPS implantation. After the procedure, the patient's urine output increased from 2300 to 5780 mL/day over the course of 5 days, and his ascites gradually decreased. Postprocedural laboratory tests showed that the patient's serum bilirubin level, liver function, and coagulopathy had improved after some time (Fig. 3). No procedure-related complications were observed after the procedure; this included encephalopathy, which was not observed after the procedure. Even so, the patient died 107 days after TIPS placement because of influenza-induced pneumonia and multiorgan failure, but the patient remained free of SFSS-related symptoms before death.
To date, there is no consensus on the definition of SFSS, but in general it has been recognized as an overperfusion of the graft by the portal vein after LDLT.8, 9 Generally, partial splenic embolization is the first-line treatment for SFSS, although surgical shunts and pharmacological agents (including somatostatin and beta-blockers) have also been reported with satisfactory results.4, 5, 10 Although TIPS placement represents a safe and effective method for the treatment of variceal bleeding and refractory ascites, its application for SFSS has been associated with mortality and morbidity.7, 11 Nonetheless, adequate results were achieved in the present case.
After transplantation, the patient quickly developed jaundice and ascites. A Doppler ultrasound examination did not detect anything unusual except for an increased portal flow rate. Biopsy showed centrilobular sinusoidal dilatation and marked lobular hepatocanalicular cholestasis, which are largely consistent with the findings of SFSS.12 The patient was diagnosed with post-LDLT SFSS, and after a conservative treatment failed, it was clear that a more effective treatment was required; thus, TIPS placement was performed 35 days after transplantation. After TIPS implantation, the portal pressure decreased from 28.7 to 15.4 mm Hg, and gradual improvements in graft function and ascites were observed. Nonetheless, because the patient died shortly after TIPS placement, the long-term effects of the procedure on SFSS could not be observed in this case.
In conclusion, TIPS creation is technically feasible for the treatment of SFSS after LDLT, and when partial splenic embolization is not feasible, TIPS may be an option for the treatment of SFSS after LDLT. However, more research is still needed to determine the effectiveness and safety of TIPS placement in the treatment of SFSS.